Stiff-Person Syndrome
Autor: | Colin D. Lambert, Anthony E. Lang, Mandar S. Jog |
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Rok vydání: | 1992 |
Předmět: |
medicine.medical_specialty
business.industry Mechanism (biology) Glutamate decarboxylase General Medicine Disease Vitiligo Bioinformatics medicine.disease Pathophysiology Endocrinology Neurology Internal medicine Diabetes mellitus Etiology Medicine Neurology (clinical) business Stiff person syndrome |
Zdroj: | Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques. 19:383-388 |
ISSN: | 2057-0155 0317-1671 |
DOI: | 10.1017/s0317167100042025 |
Popis: | The stiff-person syndrome is a disorder of persistent, painful muscle contractions predominately affecting the axial musculature. We describe a patient with this disorder and review its pathophysiology. Molecular biologic and immunologic techniques have recently added to the understanding of the mechanism of this disorder. Association with diseases such as diabetes, vitiligo and hypothyroidism have strengthened the auto-immune nature of this syndrome. Auto-antibodies against glutamic acid decarboxylase (GAD), an intraneuronal enzyme, have been implicated in the etiology of this unique disease. Therapeutic intervention with agents such as benzodiazepines that modify central GABAergic activity have demonstrated significant benefit in patients with stiff-person syndrome. |
Databáze: | OpenAIRE |
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